Fibromyalgia Online - NFA Newsletter

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Fibromyalgia Online - NFA Newsletter

Vol. 6, No.4

Unlocking the Mystery of Sjögren's Syndrome

By Bancroft

Sjögren's Syndrome Foundation

Sjögren's (SHOW-grins) syndrome, an autoimmune disease in which the

body's immune system mistakenly attacks its own moisture-producing

glands, often starts out as mystery. In fact, because of the variety

of

symptoms, it takes slightly more than six years, on average, to

diagnose

a Sjögren's syndrome patient.

Shafer was in her late 20s when she started having symptoms of

Sjögren's. She noticed occasional pain in her legs and petechiae, a

rash of red dots under the skin, on her legs. After a couple years of

intermittent symptoms, she visited her family physician and was

referred

to a rheumatologist when her blood work suggested an autoimmune

disorder.

Eventually presented one of the more tell-tale signs of

Sjögren's: dry eyes. In the end, it took about ten years to

get a diagnosis of Sjögren's syndrome.

Because diagnosis can be such a challenge, says that she would

tell people to pursue that journey of connecting with the right

provider, no matter how long it takes, no matter how many doctors you

have to go to, even if you have to spend some of your own money to do

it.

Symptoms of Sjögren's syndrome vary, but the hallmark symptoms are

dry

mouth and dry eyes. People think of saliva as a trivial substance and

tend to take it for granted, but when it is absent, it can have some

life-altering consequences, says Frederick Vivino, M.D., a

rheumatologist and director of the University of Pennsylvania

Sjögren's Syndrome Clinic in Philadelphia. Patients may have trouble

talking or may have to alter their diets to avoid spicy foods and use

gravies to moisten drier foods. Severe dental decay can also occur.

With dry eyes, people often complain of a sandy or gritty feeling.

Imagine what you feel like when your're walking on the beach on a

windy

day and can't quite get the sand out of your eyes, suggests Vivino.

Other common symptoms include dryness of the skin, lungs, vaginal

tissues, sinuses and gastrointestinal tract; joint and muscle pain;

swollen glands; chronic fatigue and numbness.

Often times Sjögren's symptoms may come and go or don't seem severe

enough to warrant a visit to the doctor. Patients may talk to their

dermatologist about a rash or their ophthalmologist about their dry

eyes, but because the physicians are not interfacing or may be

unfamiliar with Sjögren's, it may take a while for the pieces of the

puzzle to come together.

The Sjögren's Syndrome Foundation (SSF) estimates that as many as

four

million Americans have Sjögren's syndrome, which affects women nine

times more frequently than men. It's really a major women's health

problem that is largely being ignored by the medical community,

acknowledges Vivino.

In the future, I'd certainly like to see a greater emphasis in the

medical and dental school curriculums on the disease and its

manifestations.

One of the obstacles to diagnosing Sjögren's is that there is not one

simple test to identify it. Fortunately, within the past two years

there

has been a set of objective criteria developed to identify Sjögren's.

Through subjective and objective measures which include patient

interviews, blood work, a lip biopsy and tests that determine

salivary

and lacrimal (tear) function, doctors are able to determine if

patients

meet four of six criteria to make a diagnosis of Sjögren's.

The severity of symptoms among Sjögren's patients varies almost as

much as the symptoms themselves. For some, their symptoms are so

severe

that chronic fatigue or the inability for those with dry eyes to

work in

front of a computer forces them to leave their careers and go on

long-term disability. Shafer, 43, describes her condition as more of

a nuisance.

It hasn't stopped me from doing what I normally do, but, for

instance,

when I go to bed at night, I feel like I have a million things to do.

This routine includes the application of eye ointments, taking

vitamins

for her eyes and an inhaled steroid, as well as Plaquenil, an

anti-malarial medication which relieves fatigue and joint and muscle

pain in many Sjögren's patients. Meticulous tooth brushing,

flossing and fluoride applications also are important parts of the

bedtime routine.

Kim Vaughn, a 45-year-old mother of two, has had numerous health

problems since being diagnosed with Sjögren's syndrome fifteen years

ago. In addition to suffering from mini strokes, arthritis and

depression, she has had to have surgery on her sinuses as a result of

fungal infection caused by dryness, and has to have her teeth cleaned

every two to three months because of the decay caused by her dry

mouth.

" It's more than dry eyes and dry mouth. It's systemic, it affects

your

whole body, " she says.

Vaughn has not slept a full night since learning she had Sjögren's,

and finding the energy to meet the needs of her 10- and 15-year-old

sons

has been one of the hardest things for her. Every day brings its own

set of challenges, because I don't know how I am going to feel. It's

frustrating to have to take a nap in the middle of the day when there

are so many other things you could be doing. "

Vaughn, who is managing her symptoms through exercise and medication,

acknowledges that there are many promising advancements on the

horizon

for patients, but admits that knowing that she is at increased risk

of

non-Hodgkin's lymphoma scares her. Approximately one in 20 of those

with

Sjögren's syndrome will develop lymphoma, the majority non-Hodgkin's

lymphomas. Additionally, about 50 percent of Sjögren's patients have

another autoimmune disorder like lupus or rheumatoid arthritis.

The exact cause of Sjögren's is unknown but it's believed that people

inherit genetic tendencies to develop an autoimmune disease and that

environmental triggers, such as a virus, bacteria, or even chemical

exposure could start the disease process. Sjögren's is a chronic

disease, meaning that it lasts a lifetime so treatments are designed

to

manage the symptoms, not address the underlying cause.

According to dentist Dr. Philip Fox, president of the SSF, there are

now

three prescription medications approved for use with Sjögren's, which

include two drugs that stimulate salivary production and one to

improve

tear function. Other treatments include an exercise regimen,

procedures to close ducts through which tears escape to retain

moisture,

over-the-counter moisture replacement products, aspirin and other

non-steroidal anti-inflammatory drugs, steroids, and

immunosuppressant

drugs. Fox anticipates that the next five years in Sjögren's

research will be very exciting,

" In the meantime, there are a lot of simple things one can do to

minimize symptoms. he says. " These are detailed in The Sjögren's

Syndrome Handbook, a publication of the Foundation.

For more information on Sjögren's syndrome, call the Sjögren's

Syndrome Foundation at (800) 475-6473 or visit www.sjogrens.org.

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